Accuracy of [18F] fluorodopa positron emission tomography for diagnosing and localizing focal congenital hyperinsulinism

Accuracy of [18F] fluorodopa positron emission tomography for diagnosing and localizing focal congenital hyperinsulinism
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DOI:
10.1210/jc.2007-1637
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发表时间:
2007-12-01
影响因子:
5.8
通讯作者:
Stanley, Charles A.
Stanley, Charles A.
中科院分区:
医学2区
文献类型:
--
作者:
Hardy, Olga T.;Hernandez-Pampaloni, Miguel;Stanley, Charles A.

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目的:先天性高胰岛素血症(HI)婴儿的局灶性病变代表了腺瘤病的区域,该区域表达了由于母体11 p区域的胚胎杂合性丢失而导致的父系来源的ATP敏感性钾通道突变。本研究评估的准确性F-18-氟-L-二羟基苯丙氨酸([F-18]多巴)正电子发射断层扫描(PET)在诊断局灶性与弥漫性疾病和确定的位置focal lesions.Design:共50名婴儿HI无反应的药物治疗进行了研究。患者静脉注射[F-18]多巴,PET扫描50-60分钟。图像与腹部计算机断层扫描共配准。结果:50例中44例(88%)诊断为局灶性或弥漫性HI。[F-18] DOPA PET确定了24例局灶性疾病患者中18例放射性药物高摄取的局灶性区域。在所有病例中,这些病变的位置与PET扫描上[F-18] DOPA摄取增加的区域相匹配。PET扫描正确定位了5个在手术中无法可视化的病变。结论:18F-DOPA PET显像诊断局灶性腺瘤病的准确率为75%,定位准确率为100%。这些结果表明,[18 F] DOPA PET成像为手术切除局灶性腺瘤病提供了有用的指导,应考虑作为所有患有先天性HI且患有医学上无法控制的疾病的婴儿的手术指导。
Objectives: Focal lesions in infants with congenital hyperinsulinism ( HI) represent areas of adenomatosis that express a paternally derived ATP-sensitive potassium channel mutation due to embryonic loss of heterozygosity for the maternal 11p region. This study evaluated the accuracy of F-18-fluoro-L-dihydroxyphenylalanine ([F-18] DOPA) positron emission tomography ( PET) scans in diagnosing focal vs. diffuse disease and identifying the location of focal lesions.Design: A total of 50 infants with HI unresponsive to medical therapy were studied. Patients were injected iv with [F-18] DOPA, and PET scans were obtained for 50-60 min. Images were coregistered with abdominal computed tomography scans. PET scan interpretations were compared with histological diagnoses.Results: The diagnosis of focal or diffuse HI was correct in 44 of the 50 cases (88%). [F-18] DOPA PET identified focal areas of high uptake of radiopharmaceutical in 18 of 24 patients with focal disease. The locations of these lesions matched the areas of increased [F-18] DOPA uptake on the PET scans in all of the cases. PET scan correctly located five lesions that could not be visualized at surgery. The positive predictive value of [ 18F] DOPA in diagnosing focal adenomatosis was 100%, and the negative predictive value was 81%.Conclusions: [F-18] DOPA PET scans correctly diagnosed 75% of focal cases and were 100% accurate in identifying the location of the lesion. These results suggest that [ 18F] DOPA PET imaging provides a useful guide to surgical resection of focal adenomatosis and should be considered as a guide to surgery in all infants with congenital HI who have medically uncontrollable disease.